Autoimmune sensorineural hearing loss: a human temporal bone study.
OBJECTIVE
To describe histopathologic findings in temporal bones of a patient whose clinical history suggests a sensorineural hearing loss (SNHL) of autoimmune origin.


METHODS
Temporal bones from a patient with a history of ulcerative colitis, leukemia, and SNHL were examined by light microscopy.


RESULTS
Histopathologic findings included: (1) organs of Corti missing or absent in all cochlear turns; (2) cells decreased in spiral ganglia, and lymphocytic infiltration; (3) absence of portions of the spiral prominence; (4) endolymphatic hydrops in basal, middle, and apical cochlear turns and in the saccule and utricle; (5) fibrosis and osteoneogenesis of a scala tympani of the basal turn of the cochlea, the posterior semicircular canal, and the canal of Cotugno; (6) fibrosis of the vestibular aqueduct and endolymphatic sac; and (7) lymphocytes in the endolymphatic sac, perisaccular area, inferior cochlear vein, and Rosenthal's canal.


CONCLUSIONS
Histopathologic findings in the temporal bones of this patient with ulcerative colitis, sensorineural hearing loss, and vestibular symptoms closely parallel those in a previously reported animal study of autoimmunity and suggest the possibility of a SNHL of autoimmune origin.
Hoistad
D L
DL
Department of Otolaryngology, School of Medicine, University of Minnesota, Minneapolis, USA.
Schachern
P A
PA
Paparella
M M
MM
D000293:Adolescent; D001327:Autoimmune Diseases / Q000473:pathology*; D003093:Colitis, Ulcerative / Q000150:complications / Q000473:pathology; D017809:Fatal Outcome; D006319:Hearing Loss, Sensorineural / Q000150:complications / Q000276:immunology* / Q000473:pathology; D006801:Humans; D015479:Leukemia, Myelomonocytic, Acute / Q000150:complications / Q000473:pathology; D008297:Male; D013701:Temporal Bone / Q000473:pathology*; D015837:Vestibular Diseases / Q000150:complications / Q000473:pathology